Alpha thalassaemia

Description

Alpha-thalassemia is an inherited haemoglobinopathy characterised by impaired synthesis of alpha-globin chains leading to a variable clinical picture depending on the number of affected alleles, and encompassing the alpha thalassaemia trait, haemoglobin H disease (HbH) and Bart's hydrops fetalis.

Excludes

KA85 – Hydrops fetalis due to haemolytic disease

Exclusions from above levels

04 – Diseases of the immune system
05 – Endocrine, nutritional or metabolic diseases
18 – Complications of pregnancy, childbirth or the puerperium

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19 – Certain conditions originating in the perinatal period
20 – Congenital malformations, deformations or chromosomal abnormalities
22 – Injury, poisoning or certain other consequences of external causes
http://id.who.int/icd/entity/1000704511 – Human immunodeficiency virus disease
JB64.1 – Other diseases of the blood or blood-forming organs or certain disorders involving the immune mechanism complicating pregnancy, childbirth or the puerperium

Child codes

3A50.00 – Mild alpha thalassaemia diseases
3A50.01 – Thalassaemic alpha-chain variants
3A50.02 – Haemoglobin H disease (– α/– – included)
3A50.03 – Hemoglobin Bart's fetalis syndrome
3A50.0Y – Other specified alpha thalassaemia
3A50.0Z – Alpha thalassaemia, unspecified

Sibling codes

3A50.1 – Alpha thalassaemia related syndromes
3A50.2 – Beta thalassaemia
3A50.3 – Delta, delta-beta or gamma-delta-beta thalassaemia
3A50.4 – Hereditary persistence of fetal haemoglobin
3A50.Y – Other specified thalassaemias
3A50.Z – Thalassaemias, unspecified

Foundation entity

Alpha thalassaemia – http://id.who.int/icd/entity/531667506

Part of: 3A50: Thalassaemias › 03: Diseases of the blood or blood-forming organs